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Monday, June 24, 2013

Each month we visit two different doctors: a maternal fetal specialist and a pediatric cardiologist. We had our two appointments last week. Here's what we learned.

Maternal Fetal Specialist

He is basically a high risk OB. I went to this appointment with my mom so Jeff wouldn't have to take time off work, but I needed someone there in case something was not normal. At the appointment they did an ultrasound (if you are counting, that would be #7 this pregnancy) to make sure the baby is growing normally. Afterwards we talked to the doctor and asked a few questions. To be honest, we really aren't impressed with this doctor. I'm really not sure he knows much about HLHS. Some of the things he's said are not really true and we kind of get the impression that he really thinks we should terminate the pregnancy. He never actually asks us what we want to do, but he says things like, "and then we need to decide what you guys want to do... how far you want to try and go with this.". He kind of just has a "gloom and doom" kind of attitude about it. I don't think we will be seeing him again. I have to switch over to Jeff's insurance this month because I lose mine since I'm not going back to teaching. We'll see what next month brings. We did learn that the baby is growing normally (yay!) and he said that there isn't any reason to induce labor early, so I will probably deliver around my actual due date. The hospital situation is still up in the air due to the switch in insurance.

Pediatric Cardiologist

We LOVE him! If you ever find yourself in need of a pediatric cardiologist (and I hope you never do), let me know. I can recommend the best one to you. He just has the best attitude about everything. He makes us feel like, "It's okay. We've done this before. You don't need to worry about things." Not that we know everything will turn out the way we want, but worrying about things that we can't control is useless. We'll deal with the complications when they come, but for now things look okay.

The technician did another fetal echocardiogram (basically a fancier ultrasound, so #8). As she was looking at the baby we were asking her a few questions about TAPVR and Heterotaxy, but she had to stop us because she said she didn't know about all that. She was impressed with the knowledge that Jeff and I have gained already about this. That reassured us that we are on the right track with what we have learned so far.

After the echo, we met with our cardiologist. He sat down and answered every single question we had. Some of the things we talked about were:

-The baby's anatomy looks the same, which is good. I was really worried that another complication was going to pop up.

-The TAPVR is probably unobstructed, but they are going to keep watching it. These are the veins that go from his lungs to his heart. They are not connected in the right place, so eventually they will need to be fixed. They are measuring the pressure (isn't that crazy... that they can measure the pressure of the veins in utero?!) to see if it goes up or down before he is born. That will determine when they will need to address these veins.

-Our baby does not have Heterotaxy. We've heard that Heterotaxy is a common complication with the diagnosis our baby has. That is where all of his internal organs are jumbled up and not positioned correctly. The technician said it's kind of like a chain reaction, when one thing goes wrong, more things follow. But the cardiologist told us that he sees the liver, stomach, etc. in the correct positions, so yay!

-We have narrowed our hospital options down to two.

*Hospital #1 - located downtown (45-60 min. away from home), heart surgeon has worked there for 30+ years and is very good, has good accommodations for me and for the baby

*Hospital #2 - located about 4 miles away (10 min. from home), is an academic hospital so we would be working with a lot of residents which may add frustration to an already high-stress situation, has ok accommodations for me and great for the baby, this pediatric cardiology unit is ranked #12 in the nation

Obviously there are pros and cons to both. We've been assured that either place we go will have the same outcome for our baby. The 2nd hospital is actually a Primary Children's hospital, but they have added on a section where women can deliver babies there. We've been warned though, that if there was some sort of complication with delivery, there are only pediatricians there to assist. Our doctor said that 99% of the time it is fine, but he worries about the 1% of the time when it's not.

Next month, in conjunction with our cardiology appointment we will visit each hospital, meet the surgeons, and tour the facilities. I hope that helps us make our decision.

-We also asked our cardiologist about this case study at the Mayo Clinic. He actually did his residency there and said it is an amazing hospital. But he also said that he needed more information about this particular study before he would recommend it to us. He said that one of his reservations about it is that it would be very difficult to determine if it is effective. Some HLHS babies have very strong heart beats, and others have weaker heart beats. He wants to know more about how they would actually measure if the stem cells did any good, or not. He doesn't want us to do it just to do it. He said that there isn't really any added risk, but his gut feeling is that it probably won't do any good. He has emailed the hospital to get more information and we'll talk about it more once he hears back from them.

-We also discussed more about the "inter-stage" between the baby's first and second surgeries (when the baby is 0-6 months old). This is the most fragile time for HLHS babies and we've heard lots of information about how to keep the baby healthy during this time. Our doctor is a member of the same church as us, so he confirmed that we should not be taking him to church during this time or any other public place. Since he will probably be coming home from the hospital right when flu season starts he will need monthly injections to protect him from RSV. I forget what he called them, but basically it's like a monthly flu shot, but it costs $3000!! Good thing we have insurance that will pay for it!

I think that is everything. Probably pretty boring for most of you, but I wanted to write it all down before I forgot what we talked about. I'll update more after we visit the hospitals on July 22.

Monday, June 17, 2013

In the past month I have done more googling of HLHS than anything else. It has been so helpful to read other people's experiences with this. Every situation is so different, and every family has different experiences, but it is so helpful to know what we may expect. No one writes the book of "What to Expect When You're Expecting a Baby With HLHS." But I guess that's because things can go so many different ways.

Anyway, one wonderful thing I found through all my internet research (What would I do without the internet??) is the website Sisters By Heart. They are a group of HLHS heart moms who keep a blog and send care packages to support families with HLHS babies. I sent in a request for a care package a few weeks ago and didn't tell Jeff. I wanted to surprise him with something thoughtful, I guess. The package came on Friday and it was so much fun to open. They did a wonderful thing and put a small note on each item explaining why it may be helpful in the future. We loved it. It was wonderful!

Car seat sign - It says, "Please wash your hands before touching mine." This can be used once we finally get to take him out of the hospital and/or house.

Sisters By Heart Pen

a heart ring - for mom

a deck of cards - for dad to keep busy during long hours in the hospital

ZoLi bottles - I don't know much about these, but apparetly they are environmentally friendly.

canvas bag - A huge, nice bag that everything came in.

Sisters By Heart Notebook

Baby Legs - I've heard these are great for keeping your baby "dressed" in the hospital, but not interfering with all the cords, tubes, and diaper changes.

Pacifier

EZ AXS Hospital Gown - This has snaps in front and on each shoulder which can be opened to allow easy access to the baby's chest.

Zutano Side-Snap Onesie - I've heard these are great because the snaps allow tubes to easily fit through (in case he has to come home on oxygen or a feeding tube) and the snaps are on the sides instead of in front so it does not irritate chest scars. Plus, this one is super cute!

Tuesday, June 11, 2013

According to our doctors this pregnancy should go normally. I say should because if there is an exception to that rule, I now feel like it's me. With HLHS occurring in approximately 4 in 10,000 births, Jeff and I are contemplating buying a lottery ticket. Instead of seeing a normal OB doctor, I now see a Maternal Fetal Specialist for my appointments. (Hello 45 minute drive and $50 co-pay. ugh...) I have my second appointment with him this week. I'm not sure exactly what will happen at each appointment, but I assume I will be doing an ultrasound at each appointment with him. When I called the office today to see what they would be doing at my upcoming appointment, the nurse (or receptionist?) said they would be "looking at the heart." I had to laugh and hold back a sarcastic comment. So I guess I'll update after that appointment.

We also are very fortunate to get to visit with a Pediatric Cardiologist throughout my pregnancy. I consider this such a blessing because he is awesome. And he can answer so many questions for us. He is the one who cares for these babies after they are born, so he knows a little bit more about what our future holds. At our last appointment with him, he told us that our baby boy has another anomaly on top of HLHS, which is Total Anomalous Pulmonary Venous Return (TAPVR). This means that his pulmonary veins, which connect the lungs to the heart are not formed properly. At my next appointment with him he will be able to determine if these veins are obstructed or unobstructed, which will determine when our baby will need his first surgery.

Delivery

We don't know much about this day. When I asked the doctors about it at my first appointments, they said we could discuss it more in the future. I've heard that during week 25 of my pregnancy is the best time to see the baby's heart and what we're dealing with. Right now I am 24 weeks, so hopefully after next week we'll have some more answers.

What we do know is that heart babies recover better from vaginal births as opposed to c-section births. So I will hopefully be able to have a normal birth. We don't know yet where it will be or when. From what I've read about other people's experiences with this, I would assume that I will be induced so that all the right people are in all the right places when he is born. Depending on his TAPVR, he may need surgery immediately, but they are hoping to wait until day 7 or so if possible. He will go on medication right away to keep his ductus arteriosus open. This is a hole in every infant's heart that helps with circulation in the womb. After babies are born this hole closes naturally, but keeping this hole open will keep our baby boy alive. (The more I learn about this whole thing, the more amazed I am that someone knew all this and figured out how to make it work!)

So until delivery day I am a ticking time bomb. Just counting down the days until our life changes in a huge way!

After Delivery
After the baby is born he will be in the hospital for a while. His first open heart surgery (the Norwood) will take place within the first week. After that, he will be in the hospital recovering and learning to eat. This can last anywhere from 4-8 weeks. Some babies are put on feeding tubes (in fact, that used to be the norm), but our cardiologist said that we will really try and avoid that as much as possible. It causes more problems down the road. He said that kids on feeding tubes will often lose their sucking reflex and the ability to close off their wind pipe when eating. So we want to avoid that by giving him a binky as much as possible and trying to nurse him right away, even if it's just for practice.

Going Home
This is what we are aspiring to! Not every HLHS baby makes it home. I know we need to prepare ourselves for that realization, but I am also so hopeful that we will make it this far.

From what we've heard, this is the most fragile time in these babies lives. From this point until his second open heart surgery (the Glenn) which should take place 4-6 months after the baby is born, we will be the super-paranoid parents. But for good reason. A simple cold can put him back in the hospital and cause numerous complications including death. From what we've heard of other's experiences we can expect that he will not be allowed in public (church, grocery store, park, etc.). We have also heard that anytime we are in public, after we get home we should change our clothes and wash our hands before interacting with the baby. I've heard others say that they are to limit the number of times they open their front door and be very, very cautious of how many visitors they allow inside. And we will be doing all of this from November-February... right in the heart of cold and flu season. Yay.

According to our cardiologist, our baby boy should not have to be on oxygen or on a feeding tube when he comes home. But I've heard from many others that there is a good chance he might be. Especially because we are at such a high altitude. We have heard that we will be monitoring oxygen levels constantly and weight. We will need a hospital-grade scale that can measure super small increments to see if the baby is gaining or losing even a tiny amount of weight. As scary as it sounds, our doctors have assured us that all of this will give us peace of mind. We will know exactly how he is doing and exactly when something is not quite right. I guess that's a good thing. After his second surgery, life should be a bit more stable.

The third open heart surgery (the Fontan) will take place anywhere between 3-5 years old. After this surgery, life should go on "normally." It's very difficult to say what life will be like after this point, but other HLHS kids that I've read about are doing very well. They are able to run around like normal kids and play sports with their friends. They may tire more easily than their peers, but they don't have to be afraid to push themselves. They don't have to live in a bubble. We are hoping for the best for our baby boy's future.

One thing we've learned about HLHS is that it has only been treated for about 20-25 years. Which means if I would have had this condition as a baby, I would not have survived. SO MUCH has happened since then. Today, approximately 80% of these babies survive. And so much is continuing to happen.

One thing our cardiologist told us is that heart transplants are not the best way to solve this problem. Heart transplants only last about 15 years and we don't want to start the clock ticking from day 1. Plus, there's the problem of finding an infant heart. So instead it's best to treat these babies with the three surgeries which should last them maybe 20 years or so until the heart wears out. Then they will be a better candidate for a heart transplant. But, more importantly, 20 years from now there may be far better options than heart transplant.

I cam across this article today which I think foreshadows what may be in our future.

Friday, June 7, 2013

May 6 - We went in for our anatomy scan and to find out the gender of our baby. Everything looked good, we found out we were having a BOY, but when it came time to look at the heart, the ultrasound tech got very quiet. After searching for a while she told us to come back in a week because she couldn't see the heart very well. As we left the appointment I was feeling a little worried, but Jeff assured me that everything was fine.

May 13 - We went back for some more pictures of the baby's heart. After searching for half an hour or more, a second ultrasound technician came in. She looked around, took some pictures and told us that she would send them to my doctor and we would, "go from there." I left that appointment feeling very worried. Jeff again assured me that everything was fine. I tried not to stress about something I didn't know for sure, but I had a feeling that something was not right.

May 15 - After worrying for two days straight and waiting on pins and needles to get a phone call from my doctor I finally started to relax a little bit. I kept telling myself that if it was something serious, they would have called me sooner. But that afternoon, I got the phone call from my doctor. She told me they noticed an "underdeveloped left ventricle" in the baby's heart and that I needed to make an appointment with a maternal fetal specialist. I hung up the phone and immediately started googling the phrase "underdeveloped left ventricle." I know the internet is not super trustworthy and it can be very scary, but I needed more information of some kind. I also called the maternal fetal specialist and made an appointment for next morning at 8:00 am.
Jeff left work early to come home so we could be together and my parents and sister's family came over that evening. We talked about what the possibilities were. Jeff, my dad, and I had all goggled on our own and noticed that HLHS (Hypoplastic Left Heart Syndrome) came up over and over again. I was pretty sure that this is what we were facing, but still very scared about the unknown. My dad gave Jeff and I a blessing. My mom and dad offered to take us to the appointment the next morning (which was an hour away).
What I mostly remember from that night is being very, very scared about the unknown. Scary questions and thoughts were very difficult to push from my mind because we basically knew nothing at this point.

May 16 - We met with the maternal fetal specialist. They did another full body ultrasound to see if there was anything else going on besides heart issues. And of course they looked very closely at the heart again. After the ultrasound was finished the specialist talked to us and told us that the official diagnosis would be "Hypoplastic Left Heart Syndrome." He could not answer a whole lot more questions because he deals mainly with the pregnancy side of things. As far as pregnancy, everything should go normally. I don't have to be on bed rest. I just need to concentrate on growing this baby as big a possible so they have more heart to work on after he is born. At this point there is nothing we/I can do to make the problem better or worse. They set up an appointment for us with a cardiologist for the very next day at 9:00 am.
I remember leaving the appointment with a mix of so many emotions. Grief. Fear. Uncertainty. Confusion. Peace. The rest of the day was spent is a daze with a roller coaster of emotions constantly coming and going.

May 17 - We met with our cardiologist. They did another ultrasound (That's 4 in two weeks, if anyone's counting.) and a fetal echocardiogram. This took over an hour. After taking many, many pictures of the baby's heart, she took them to show the cardiologist. We waited in a small room nearby for almost an hour. At times we thought maybe they forgot about us, but then they would stop by to offer snacks. The cardiologist popped in for a second while I was in the restroom to say that he had someone else to talk to and then he'd be back. He wanted to clear his schedule for us because "this will not be a short conversation."
When he came back he talked us through our diagnosis. He was very good about explaining everything that should be happening in a healthy heart and how our baby's heart is different. He also told us that our baby has HLHS and another anomaly, TAPVR (Total Anomalous Pulmonary Venous Return) that we will need to deal with. He talked us through the three open heart surgeries that will take place after the baby is born. And he answered the many, many questions we had.
We were so blessed to meet this cardiologist. We feel like we are in such good hands. I left this appointment feeling a lot more peace and physically stronger than the days before. I felt like we finally had some knowledge on this subject and knowledge is power. Now that we knew what we were up against we could start moving forward.

Now - Every since that crazy week Jeff and I have been doing better and better. Sometimes the emotions from all of this catch up with us. Sometimes the stress of it all hits hard. But for the most part we are doing well. We are happy. This is the trial that our Heavenly Father has given us, and we are doing our best to endure it well.

Your love, prayers, thoughts, and words of encouragement are very much appreciated. Many people have reached out to us and we are so blessed to have such wonderful friends and family. We love you!!

I have to preface this by first saying that I know I am a horrible blogger. I haven't updated our family blog in over a year and I don't think that is going to change. But I do have three specific reasons why I wanted to start another blog.

1. After we received the news about our precious baby boy, I got so much peace and comfort from reading other people's blogs who have been down this path. I still read blogs from these families and love to hear about their experiences. Every child with HLHS is a little different so it is nice to get some kind of an idea about what is in our future. So I guess I am doing this to "pay it forward" to other heart families in the future.

2. There are many family members and friends that we want to keep informed about things and not enough time to call them all. Plus, passing messages from one person to the next can get tiring and confusing. So we are doing this to keep others informed.

3. I have already heard from other heart families that so much happens so fast with HLHS babies that they almost forget what they have been through and how far they have come. So I am also doing this to keep a record for ourselves.